title: Geosmithia argillacea: an emerging cause of invasive mycosis in human chronic granulomatous disease.
abstract: BACKGROUND
  Chronic granulomatous disease (CGD) is an inherited disorder of the nicotinamide adenine dinucleotide phosphate oxidase that leads to defective production of microbicidal superoxide and other oxidative radicals, resulting in increased susceptibility to invasive infections, especially those due to fungi.
  
  
  METHODS
  Geosmithia argillacea was identified from cultured isolates by genomic sequencing of the internal transcribed spacer region. Isolates previously identified as Paecilomyces variotii, a filamentous fungus closely resembling G. argillacea, were also examined.
  
  
  RESULTS
  We identified G. argillacea as the cause of invasive mycosis in 7 CGD patients. In 5 cases, the fungus had been previously identified morphologically as P. variotii. All patients had pulmonary lesions; 1 had disseminated lesions following inhalational pneumonia. Infections involved the chest wall and contiguous ribs in 2 patients and disseminated to the brain in 1 patient. Four patients with pneumonia underwent surgical intervention. All patients responded poorly to medical treatment, and 3 died.
  
  
  CONCLUSIONS
  We report the first cases of invasive mycosis caused by G. argillacea in CGD patients. G. argillacea infections in CGD are often refractory and severe with a high fatality rate. Surgical intervention has been effective in some cases. G. argillacea is a previously underappreciated and frequently misidentified pathogen in CGD that should be excluded when P. variotii is identified morphologically.
authors.affiliation: Laboratory of Host Defense, National Institutes of Allergy and Infectious Diseases, Bethesda, MD, USA. sderavin@niaid.nih.gov
authors.forename: Suk See
authors.initials: SS
authors.lastname: De Ravin
authors.forename: Malliswari
authors.initials: M
authors.lastname: Challipalli
authors.forename: Victoria
authors.initials: V
authors.lastname: Anderson
authors.forename: Yvonne R
authors.initials: YR
authors.lastname: Shea
authors.forename: Beatriz
authors.initials: B
authors.lastname: Marciano
authors.forename: Dianne
authors.initials: D
authors.lastname: Hilligoss
authors.forename: Martha
authors.initials: M
authors.lastname: Marquesen
authors.forename: Rosamma
authors.initials: R
authors.lastname: Decastro
authors.forename: Yen-Chun
authors.initials: YC
authors.lastname: Liu
authors.forename: Deanna A
authors.initials: DA
authors.lastname: Sutton
authors.forename: Brian L
authors.initials: BL
authors.lastname: Wickes
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Kammeyer
authors.forename: Lynne
authors.initials: L
authors.lastname: Sigler
authors.forename: Kathleen
authors.initials: K
authors.lastname: Sullivan
authors.forename: Elizabeth M
authors.initials: EM
authors.lastname: Kang
authors.forename: Harry L
authors.initials: HL
authors.lastname: Malech
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
authors.forename: Adrian M
authors.initials: AM
authors.lastname: Zelazny
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D021821:Communicable Diseases, Emerging / Q000453:epidemiology* / Q000382:microbiology*; D004271:DNA, Fungal / Q000737:chemistry / Q000235:genetics; D021903:DNA, Ribosomal Spacer / Q000737:chemistry / Q000235:genetics; D032641:Eurotiales / Q000145:classification / Q000235:genetics / Q000302:isolation & purification*; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D008297:Male; D008969:Molecular Sequence Data; D009181:Mycoses / Q000453:epidemiology* / Q000382:microbiology*; D017422:Sequence Analysis, DNA
chemical_list: D004271:DNA, Fungal; D021903:DNA, Ribosomal Spacer